Diaphragm pacing and independent breathing in individuals with severe Pompe disease

• Main Authors: Cristina Liberati (Author), Barry J. Byrne (Author), David D. Fuller (Author), Chasen Croft (Author), Teresa Pitts (Author), Jessica Ehrbar (Author), Carmen Leon-Astudillo (Author), Barbara K. Smith (Author)
• Format: Knjiga
• Izdano: Frontiers Media S.A., 2023-07-01T00:00:00Z.
• Teme: article
• Online dostop: Connect to this object online.

IntroductionPompe disease is an inherited disease characterized by a deficit in acid-α-glucosidase (GAA), an enzyme which degrades lysosomal glycogen. The phrenic-diaphragm motor system is affected preferentially, and respiratory failure often occurs despite GAA enzyme replacement therapy. We hypoth...